Complex V activity
| Patients | Muscle | Fibroblasts | Heart | Brain |
|---|---|---|---|---|
| Ped A IV/2 | 65 | 35 | 14 | 5.2 |
| Ped B III/1 | 121 | 67 | — | — |
| Ped C III/1 | 67 | 69 | — | — |
| Ped C II/1 | — | 63 | — | — |
| Ped D III/1 | 110 | 54 | — | — |
| Ped E II/1 | 90 | — | — | — |
| Normal values | 154 (21) | 106 (25) | 57 (20) | 18.4 (3.6) |
Values are means (SDs) expressed as nmol/min/mg of protein as a ratio with citrate synthase X 100.